Cases in Adult Congenital Heart Disease

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1 Cases in Adult Congenital Heart Disease Sabrina Phillips, MD FACC FASE Associate Professor of Medicine The University of Oklahoma Health Sciences Center

2 No Disclosures

3 I Have Palpitations

4 18 Year old Man Palpitations abnormal ecg and cxr

5 ECHO

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14 Ebstein Anomaly

15 Normal Delamination of the TV from the RV Myocardium

16 Failure of Delamination From the Myocardium Spectrum with Infinite Variability

17 Failed Delamination results in adherence of leaflets to underlying RV myocardium displacement of the anular hinge points

18 Displacement Apically AND Toward the Right Ventricular Outflow Tract

19 Echocardiographic Diagnosis Apical displacement of the septal leaflet of the tricuspid valve > 8mm/m2 Right sided chamber enlargement with atrialized RV Tricuspid valve regurgitation often appears laminar Elongated, tethered anterior TV leaflet

20 Ebstein Anomaly Associated Lesions Secundum ASD RV outflow tract obstruction LV non-compaction Accessory pathways

21 Ebstein Anomaly Indications for Operation symptoms, exercise tolerance, cyanosis progressive RV dilatation before significant RV dysfunction onset, progression of atrial arrhythmias? earlier operation if TV repair is likely prior to LV dysfunction

22 I Have a Headache

23 36 Year Old Man Undergoing evaluation in neuro for headache Found to be hypertensive

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27 Is This Coarctation? A. Yes B. No C. Not Sure

28 Is This Coarctation? A. Yes B. No C. Not Sure

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30 Abdominal Aorta Doppler

31 Abdominal Aortic Doppler Significant Coarctation Normal

32 Imaging of Coarctation of the Aorta Abdominal aorta Doppler Suprasternal notch imaging Parasternal short axis -?BAV Parasternal long axis ascending aortic dimension

33 Discrete Coarctation

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37 Coarctation Caveats Doppler gradient through the coarctation may be low 2 collaterals Abdominal Doppler pattern is critical Continuous flow in the thoracic aorta is helpful Don t forget association to BAV

38 Abnormal ECG on Life Insurance Exam

39 31 year old man Told as a child he had a hole in his heart no intervention

40 EKG

41 ECHO IMAGES

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48 Diagnosis? A. Non-compaction cardiomyopathy B. L-TGA C. D-TGA D. Ebstein anomaly

49 Diagnosis? A. Non-compaction cardiomyopathy B. L-TGA C. D-TGA D. Ebstein anomaly

50 Congenitally Corrected Transposition

51 Complete Transposition (D-TGA) Congenitally Corrected Transposition (L-TGA) RA LA RA LA AO PA PA AO RV LV LV RV

52 Conus present in the LVOT

53 Left A-V valve displaced apically

54 Side-by-side semi-lunar valves

55 Lesions Associated with cctga Ventricular Septal Defect (70%) Subpulmonary ventricular outflow tract obstruction (40%) Tricuspid valve dysplasia/ebstein malformation (90%) Situs Inversus Dextrocardia

56 Second Opinion

57 38 Year Old Woman Present for second opinion re: treatment of pulmonary hypertension Significantly limited Marked cyanosis

58 Past Medical History Evaluated at 3 months of age for pneumonia Diagnosed with VSD, PDA, coarctation PA banding, PDA ligation and coarctation repair performed

59 Past Medical History 6 years: diagnosed with Eisenmenger syndrome Treated with frequent phlebotomy Placed on Coumadin in adulthood Placed on the heart/lung transplant list 5 years (elsewhere) No birth control being used

60 Current Exam Significant cyanosis Conjunctival injection 2+ RV impulse, normal LV impulse 3/6 systolic crescendo-decrescendo murmur left upper sternal border No diastolic murmur

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71 PWD velocity = 0.5 m/s

72 CWD velocity = 4.1 m/s

73 Non-imaging Doppler velocity = 4.5 m/s

74 Does This Patient Have Eisenmenger s Syndrome? A. Yes B. No

75 Does This Patient Have Eisenmenger s Syndrome? A. Yes B. No

76 Non-imaging Doppler velocity = 4.5 m/s

77 Cath Tight PA band in appropriate location without distortion of the pulmonary valve Distal PA pressure 35/11 Band gradient: 80 mmhg Pulmonary blood flow < 1 L/min/m2 No residual coarctation No PDA

78

79 Outcome Successful PA debanding and VSD closure Transient post-op reperfusion lung injury Returned for 6 month follow-up: room air sat 95%. Normal 6 minute walk. RVSP: 51 mmhg Discontinued disability and began a new job

80 Teaching Points A VSD with a bidirectional shunt Eisenmenger syndrome look for obstruction to RV outflow causing RV hypertension Patients with Eisenmenger VSD do not have loud systolic ejection murmurs Review cath reports carefully with your interventionalist communication between the care team is essential

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